Nephrotic Syndrome with Crescent Formation and Massive IgA Deposition and Hypothyroidism following Hematopoietic Stem Cell Tra

We describe herein a case of nephrotic syndrome (NS) following allogeneic bone marrow transplantation (allo-BMT) for natural killer cell leukemia/lymphoma. Histological studies defined the diagnosis as crescentic glomerulonephritis with massive IgA deposition, which have never been reported in NS cases following allo-BMT. A majority of the massive infiltrated cells in the interstice were CD3(+)CD4(-)CD8(+) T cells derived from the donor. Mesangial deposition of Haemophilus parainfluenza outer membrane (OMHP) antigen and decreased glycosylation of the IgA1 hinge in the recipient’s samples, with which we recently reported on the pathogenesis of IgA nephropathy and its association, were observed. Further, IgA antibody against OMHP titer of the donor serum was as high as other IgA nephropathy cases. These findings suggested that NS, crescentic glomerulonephritis, of this case was caused as one of the forms of chronic GVHD and IgA deposition was associated with Haemophilus parainfluenza and decreased glycosylation of the IgA1 hinge.